Treatment of severe acquired haemophilia A with an immunosuppressive agent

Željka Škunca, Ana Planinc Peraica

Abstract


Acquired haemophilia A (AHA) is a rare hemorrhagic disease caused by an autoantibody against coagulation factor VIII. Nonhaemophiliac patients develop autoantibodies (inhibitors) directed against the factor VIII circulating coagulation protein. Disease is associated with an increased morbidity and mortality. Inhibitors against FVIII induce acute and life-threatening hemorrhagic diathesis because of abnormal blood clotting. FVIII inhibitors demonstrate bleeding disorders and prolonged activated partial thromboplastin time and a normal prothrombin time. AHA should be considered in the differential diagnosis particularly in postpartum women and in the elderly patients with bleeding tendency. Treatment of acute hemorrhage is focused in the control of the acute bleeding episode and the long term suppression of the autoantibody. In congenital hemophilia A with inhibitors, in which using repetitive infusions of high dose FVIII concentrates is effective for inhibitor eradication.

This report present one patient treated with immunosuppressive regimens. The most effective first-line treatment for the eradication of factor VIII autoantibodies is the combination of steroides and cyclophosphamide.


Keywords*


Acquired haemophilia A, Factor VIII inhibitors, treatment

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